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Respiratory Medicine Midterm > The Lung Part 6 > Flashcards

The Lung Part 6 Flashcards

1
Q

Pulmonary Eiosinophilia

A

-infiltration of eiosinophils recruited by IL-5

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2
Q

Pulmonary eiosinophilia divided into 3 categories:

A
  • Acute eiosinophilic pneumonia with respiratory failure
  • Secondary eosinophilia
  • Idiopathic chronic eosinophilic pneumonia
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3
Q

Acute eiosinophilic pneumonia with respiratory failure

A
  • acute illness; unknown ause
  • rapid onset
  • fever, dyspnea, hypoxemic resp failure
  • chest xray: diffuse infiltrates and bronchoalveolar lavage fluid contains more than 25% eiosinophils
  • Histo: diffuse alveolar damage and many eiosinophils
  • PROMPT RESPONSE TO CORTICOSTEROIDS
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4
Q

Secondary eiosinophilia

A
  • occurs in many parasitic, fungal and bacterial infxns
  • also occurs in HS pneumonitis, drug allergies, asthma, allergic bronchopulmonary aspergillosis, or vasculitis (Churg-Strauss syndrome)
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5
Q

Idiopathic chronic eiosinophilic pneumonia

A
  • focal areas of cellular consolidation of lung substance distributed in periphery of lung fields–here see aggregates of lymphocytes and eosinophils within both septal wall and alveolar spaces
  • Interstitial fibrosis and organizing pneumonia present
  • pts have cough, fever, night sweats, dyspnea, weight loss–all respond to corticosteroids!!
  • Dx of exclusion of other causes of pulm eosinophilia
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6
Q

Smoking related diseases can be divided into

A
  • obstructive diseases (emphysema and chronic bronchitis)

- restrictive or interstitial disease

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7
Q

Smoking and idiopathic pulmonary fibrosis

A
  • majority of people with idiopathic pulmonary disease are smokers but role of cigarette smoking not clear yet
  • Desquamative interstitial pneumonia and resp bronchiolitis associated interstitial lung disease also smoking associated interstitial lung diseases
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8
Q

Desquamative interstitial pneumonia

A
  • LOTS of m-phages in airspaces in current/past smoker

- previously macrophages were thought to be desquamative pneumocytes

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9
Q

Desquamative interstitial pneumonia morphology

A
  • Large number of macrophages with abundant cytoplasm with dusty brown pigment (smoker’s macrophages!!) in airspaces
  • Finely granular iron seen in m-phage cytoplasm
  • some m-phages have lamellar bodies (made of surfactant) with phagocytic vacuoles derived from necrotic type II pneumocytes
  • thickened alveolar septa by sparse inflammatory infiltrate of lymphocytes, plasma cells and occasional eiosinophils
  • septa lined by plump, cuboidal pneumocytes
  • Interstitial fibrosis when present is mild; emphysema often present
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10
Q

Desquamative interstitial pneumonia clinical course

A
  • 4th or 5th decade
  • equally common in men and women
  • ALL PATIENTS ARE SMOKERS
  • S/S: gradual onset of dyspnea and dry cough over weeks or months, associated with clubbing of digits
  • Pulm fnx test show mild restrictive abnormality w/moderate reduction of diffusing capactiy of CO2
  • Excellent response to STEROIDS and smoking cessation but few pts progress to interstitial fibrosis
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11
Q

Respiratory Bronchiolitis associated interstitial lung disease

A
  • chronic inflammation and peribronchiolar fibrosis
  • seen in SMOKERS
  • see pigmented intraluminal macrophages within first and second order resp bronchioles
  • mild form=incidental finding in smokers/ex smokers
  • term used for patients who develop significant pulm symptoms, abnormal pulm function and imaging abnormalities
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12
Q

Respiratory Bronchiolitis associated interstitial lung disease morphology

A
  • patchy at low magnification with bronchiocentric dist.
  • resp bronchioles, alveolar ducts and peribronchiolar spaces contain aggregates of dusty brown macrophages (smokers’ macrophages) similarly seen in desquamative interstitial pneumonia
  • patchy submucosal and peribronchiolar infiltrate of lymphocytes and histiocytes; mild peribrochiolar fibrosis also seen which expands contiguous alveolar septa
  • Centrilobular emphysema common but not severe
  • Desquamative interstitial pneumonia found in different parts of same lung
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13
Q

Respiratory Bronchiolitis associated interstitial lung disease clinical course

A
  • mild symptoms of gradual onset of dyspnea and cough
  • 4th or 5th decade; current smoker w/avg exposure of 30 pack years
  • smoking cessation results in improvement
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14
Q

Pulmonary Langerhans Cell Histiocytosis

A
  • rare
  • focal collections of langerhans cells (with eiosinophils)
  • As lesions progress, see scarring leading to airway destruction and alveolar damage resulting in irregular cystic spaces
  • Chest image: cystic and nodular abnormalities
  • Langerhans cell=immature dendritic cells with grooved, indented nuclei and abundant cytoplasm; positive for S100, CD1a, CD207 (langerin); negative for CD68!
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15
Q

Pulmonary Langerhans Cell Histiocytosis Clinical course

A
  • young adult smoker
  • get better after smoking cessation suggesting it might be reactive inflammatory process
  • other cases, the Langerhan cells have acquired activating mutations in SERINE/THREONINE kinase BRAF (also seen in neoplastic process and other Langerhan proliferations of other tissue)
  • neoplastic basis may explain why disease progresses in some patients even requiring lung transplant
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16
Q

Pulmonary alveolar proteinosis

A
  • Rare
  • cause: defects in GM-CSF or pulm m-phage dysfnx resulting in accumulation of surfactant in intra-alveolar and bronchiolar spaces
  • PAP characterized radiologically by bilateral patchy asymmetric pulm opacifications
  • 3 distinct classes: Autoimmune (acuired), secondary and congenital–similar histological changes
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17
Q

Autoimmune pulmonary alveolar proteinosis

A
  • caused by circulating neutralizing Abs specific for GM-CSF
  • mostly in adults; NO familial predisposition
  • knockout in GM-CSF gene in mice induces PAP and cured after tx with GM-CSF
  • Loss of GM-CSF signaling blocks terminal differentiation of alveolar macrophages impairing their ability to catabolize surfactant
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18
Q

Secondary PAP

A
  • uncommon
  • associated with hematopoietic disorders, malignancies, immunodeficiency disorders, lysinuric protein intolerance (inborn error of AA metabolism), acute silicosis, other inhalational syndromes
  • impair macrophage maturation or function leading to inadequate clearance of surfactant from alveolar space
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19
Q

Heriditary PAP

A
  • extremely rare!
  • occurs in neonates
  • cause: mutations that disrupt genes involved in GM-CSF signaling (GM-CSF and GM-CSF receptor gene mutations)
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20
Q

Morphology of pulmonary alveolar proteinosis

A
  • homogenous, granular precipitate with surfactant proteins in alveoli causing focal to confluent consolidation of large areas of lung with minimal inflammatory reaction
  • results in marked increase in size and weight of lung
  • alveolar precipitate is PAS positive with cholestrol clefts and surfactant proteins
  • surfactant lamellae in type II pneumocytes are normal
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21
Q

Pulmonary alveolar proteinosis clinical course

A
  • pt presents with cough, abundant sputum that contains chunks of gelatinous material
  • symptoms last years with febrile illnesses
  • at risk for secondary infections
  • Progressive dyspnea, cyanosis and resp. insufficiency can occur but other pts have benign course with resolution of lesions
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22
Q

Pulmonary alveolar proteinosis treatment

A
  • Whole lung lavage is standard of care and is beneficial regardless of underlying defect
  • GM-CSF therapy=safe and effective in more than half of patients with autoimmune PAP while therapy directed at underlying disorder helpful in secondary PAP
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23
Q

Surfactant Dysfunction disorders

A
  • diseases caused by mutations in genes encoding proteins involved in surfactant trafficking or secretion
  • Mutated genes include: ATP binding cassette protein member 3 (ABCA3), surfactant protein C, Surfactant protein B
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24
Q

ATP binding cassette protein member 3 (ABCA3)

A
  • most frequently mutated gene in surfactant dysfunction disorders
  • Autosomal recessive
  • presents in first few months of life
  • Rapidly progressive resp failure followed by death
  • Less commonly seen in older children and adults with chronic interstitial lung disease
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25
Q

Surfactant protein C

A
  • second most commonly mutated gene in surfactant dysfunction disorders
  • Autosomal dominant w/variable penetrance /severity
  • highly variable course
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26
Q

Surfactant protein B

A
  • least commonly mutated gene in surfactant dysfunction disorders
  • Autosomal recessive
  • usually infant is full term and rapidly develops progressive resp distress shortly after birth; death bw 3-6 months of age
27
Q

Morphology of Surfactant dysfunction disorders

A
  • variable amount of intra-alveolar pink granular material, type II pneumocyte hyperplasia, interstitial fibrosis and alveolar simplification
  • show lack of surfactant proteins C and B in their respective deficiencies
  • abnormalities in lamellar bodies in type II pneumocytes seen in all 3
  • small lamellar bodies w/electron dense cores diagnostic for ABCA3 mutation
28
Q

Diseases of vascular origin

A
  • Pulmonar embolism and infarction
  • Pulmonary HTN
  • Diffuse Pulmonary Hemorrhage syndromes
29
Q

Pulmonary embolism

A
  • bedridden patient, hypercoagulable state
  • Blood clots that occlude large pulm arteries are always embolic in origin
  • usual source=thrombi in deep veins of leg
  • associated with severe burns, trauma, fractures?
  • large vessel pulmonary thromboses are rare and only develop in presence of pulm HTN, pulm atherosclerosis and HF
  • 10% of hospital deaths
30
Q

Pulmonary embolism usually occurs in patients with

A
  • predisposing condition that produces an increased tendency to clot (thrombophilia)
  • pts usually have cardiac disease or cancer or have been immobilized for several days/weeks prior to symptomatic embolism development
  • hip fracture pts at high risk
31
Q

Hypercoagulable states associated with PE

A
  • Primary: factor V Leiden, prothrombin mutations, antiphospholipid syndrome
  • Secondary: obesity, recent surgery, cancer, oral contraceptive use, pregnancy)
  • Indwelling central venous lines can lead to right atrial thrombi which can embolize to lungs
32
Q

Rarely, pulmonary embolism may consist of

A
  • fat, air, or tumor

- Small bone marrow emboli often seen in patients who die after chest compressions for resuscitative efforts

33
Q

Two dangerous consequences of pulmonary embolism

A
  • 1) Respiratory compromise: due to nonperfused, but ventilated, segment
  • 2) Hemodynamic compromise: due to increased resistance to pulmonary blood flow caused by embolic obstruction
  • sudden death often ensues as a result of blockage of blood flow through lungs
  • death may also be caused by acute RHF (acute cor pulmonale)
34
Q

PE morphology

A
  • large emboli logde in main pulm a or its major branches or at bifurcation as saddle embolus
  • smaller emboli travel into peripheral vessels causing hemorrhage or infarction
  • if CV fnx is adequate, bronchial a supplys parenchyma–hemorrhage may occur but infarction does not
35
Q

PE–when emboli cause infarction, which parts of lungs are affected?

A
  • mostly affect lower lobes with multiple lesions
  • extend into periphery of lung as a wedge with apex pointing towards hilus of lung
  • occluded vessel identified near apex of infarct
36
Q

Pulmonary embolism distinguished from postmortem clot by the presence of

A

-Lines of Zahn in the thrombus

37
Q

Pulmonary infarct is classically

A
  • hemorrhagic and appears as a raised, red-blue area in early stages
  • pleural surface is covered by fibrinous exudate
  • red cells begin lysing within 48 hours and infarct becomes paler and eventually red-brown as hemosiderin is produced
  • With time, fibrous replacement begins at margins as gray-white peripheral zone and converts infarct into contracted scar
38
Q

Histologically hemorrhagic area in pulmonary infarct shows

A
  • ischemic necrosis of alveolar walls, bronchioles and vessels
  • If infarct is caused by infected embolus, neutrophilic inflammatory reaction can be intense–referred to as septic infarcts–some which turn into abscesses
39
Q

Clinical course of pulmonary embolus

A
  • virtually instantaneous death
  • electromechanical dissociation–ECG has rhythm but no pulse bc no blood entering pulm arterial circulation
  • big PE: can mimick MI w/severe chest pain, dsypnea, shock
  • SMALL embol: silent or induce transient chest pain and cough
40
Q

Pulmonary infarcts manifest as

A

dyspnea, tachypnea, fever, chest pain, cough and hemoptysis

-overlying fibrinous pleuritis may produce pleural friction rub

41
Q

Chest radiograph findings of PE

A
  • variable

- can be normal or disclose pulmonary infarct usually 12-36 hrs after occuring as WEDGE SHAPED INFILTRATE

42
Q

Diagnosis of PE usually made with

A

spiral computed tomographic angiography

-Rarely, other methods like ventilation-perfusion scanning or pulmonary angiography required

43
Q

Deep vein thrombosis can be diagnosed with

A
  • duplex ultrasonography
  • After acute insult, embli resolve via contraction and fibrinolysis esp in the young
  • If unresolved, over time, multiple small emboli can lead to pulm HTN and chronic cor pulmonale
  • small embolus may be warning that there may be larger one; one increases risk for second one
44
Q

Pulmonary embolism treatment

A

-Prophylactic therapy: early ambulation, elastic stockings, graduated compression stockings, anticoagulation
Treatment: anticoagulation; thrombolysis may have some benefit in pts with severe complications (shock) but carries high risk of bleeding
-Tx w/inferior vena cava filter (catches clots before reaching lung) for pts at risk of recurrent PE where anticoagulation is contraindicated

45
Q

Pulmonary Hypertension

A

-mean pulm artery pressure > or = to 25mm Hg at rest;

46
Q

Five types of pulmonary HTn

A

1) Pulmonary arterial HTN–impact small pum muscular arteries
2) pulm HTN secondary to LHF
3) pulm HTN from lung parenchymal dz or hypoxemia
4) chronic thromboembolic pulm HTN
5) pulm HTN of multifactorial basis

47
Q

Pathogenesis of pulm HTN

A

-most frequently associated with structural cardiopulm conditins that increase pulm blood flow, pulm vascular resistance, or left heart resistance to blood flow

48
Q

Most common causes of Pulm HTN

A
  • Chronic obstructive or interstitial lung dz (3)
  • Antecedent congenital or acquired heart dz (2)
  • Recurrent thromboemboli (4)
  • Autoimmune dz (1)
  • Obstructive sleep apnea (3)
49
Q

Chronic obstructive or interstitial lung dz and pulm HTN

A

-obliterates alveolar capillaries, increasing pulm resistance to blood flow and secondarily pulm blood pressure

50
Q

Antecedent congenital or acquired heart dz and pulm HTN

A

-Mitral stenosis, for example, causes increase in left arterial pressure and pulm venous pressure that is transmitted to arterial side of pulm vasculature leading to HTN

51
Q

Recurrent thromboemboli and pulm HTN

A

-reduces functional cross-sectional area of pulm vascular bed leading to increase in pulm vascular resistance

52
Q

Autoimmune diseases and pulm HTN

A

-many diseases (esp sclerosis) involve pulm vasculature and/or interstitium leading to increased vascular resistance and pulm HTN

53
Q

Obstructive sleep apnea and pulm HTN

A
  • associated wtih obesity and hypoxemia

- significant contributor to dev of pulm HTN and cor pulmonale

54
Q

idiopathic pulm arterial HTN

A
  • pulm HTN in which all known causes of pulm HTN are excluded but 80% of idiopathic pulm HTN has genetic basis!
  • sometimes autosomal dominant; incomplete penetrance
55
Q

Mutations associated with pulm HTN

A

-Inactivating germline mutations of BMPR2 in familial pulm HTN and some sporadic cases; BMPR2 down-regulated in some idiopathic pulm arterial HTN patients w/o mutation in gene

56
Q

BMPR2 protein

A
  • cell surface protein in the TGF-B family
  • binds TGF-B, BMP, activin, inhibin
  • BMP-BMPR2 important for bone growth, embyogenesis, apoptosis, cell proliferation/diff
  • haploinsufficiency for BMPR2 leads to dysfunction and proliferation of endothelial cells and vasc sm m cells
  • env triggers also contribute to pathogenesis–two hit model where genetically susceptible person w/MBPR2 mutation requires additional genetic and env insults to develop pulm HTN
57
Q

All forms of pulm HTN are associated with what morphologically?

A
  • medial hypertrophy of pulmonary muscular and elastic arteries
  • pulmonary arterial atherosclerosis
  • right ventricular hypertrophy!!
58
Q

How to determine cause of pulm HTN morphologically

A
  • presence of many organizing thrombi=recurrent PEs

- diffuse pulm fibrosis or severe emphysema and chronic bronchitis=chronic hypoxia

59
Q

Vessel changes in pulm HTN

A
  • can involve entire arterial tree from pain pulm arteries to arterioles
  • In most sever cases, atheromatous deposits form in pulm artery and its major branches resembling systemic atherosclerosis
60
Q

In pulm HTN, the ___ and ____ are most prominently affected by striking medial hypertrophy and intimal fibrosis, sometimes narrowing lumens to pinpoint channels

A

-arterioles and small arteries

61
Q

Plexiform lesion in pulm HTN

A
  • tuft of capillry formation is present, producing network or web that spans lumens of dilated thin-walled, small arteries and may extend outside vessel
  • most prominent in idiopathic and familial pulm HTN, unrepaired congenital heart dz, and pulm HTN associated with HIV and drugs
62
Q

Clinical course of pulm HTN

A
  • Idiopathic pulm HTN most common in women, 20-40yr
  • occasionally in young children
  • S/S only apparent in advanced disease: dyspnea, fatigue, can have chest pain-anginal type
  • overtime–severe resp distress, cyanosis, RVH, death from decompensated cor pulmonale often w/superimposed thromboembolism and pneumonia
63
Q

Treatment of pulm HTN

A
  • depends on underlying cause
  • in those with secondary disease, therapy directed at trigger (thromboembolic dz or hypoxemia)
  • vasodilators=varying success for group 1/refractory dz
  • Lung transplant definitive treatment

Respiratory Medicine Midterm (17 decks)

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